Central mucoepidermoid carcinoma (MEC) is a rare neoplasm of the jaw which is more common in the posterior of mandible, and the most likely origin is odontogenic epithelium. The aim of the present report is to describe a case of maxillary MEC arising in a dentigerous cyst in a 57-year-old female. It is crucial for pathologists to aware of the histopathologic features of this rare neoplasm to have a correct diagnosis.